status of pediatric cardiology at a tertiary center in Lebanon

Cardiac disease, both congenital and acquired, contributes significantly to morbidity and mortality in children. This article describes the status of pediatric cardiology at the Children's Heart Center in the American University of Beirut Medical Center. It addresses the available clinical services as well as the research and educational activities that are present at a tertiary center in Lebanon, a developing country with a population of 4 million. Lebanon has witnessed major developments in the field of pediatric cardiology over the past few years. About 650 babies are born with heart disease every year, with more than 425 needing treatment. Nearly all types of interventional catheterization procedures are currently being performed. About 300 open and closed pediatric cardiac surgeries are performed per year in Lebanon. In 2008, the in-hospital surgical mortality rate at our center was 2.6%, reflecting the good level of care in the treatment of children with congenital heart disease [CHD] in Lebanon. Basic research in the field of pediatric cardiology is emerging at our center. Our team has been studying the effect of chronic hypoxemia on the neonatal myocardium in an animal model of chronic hypoxia, as well as the study of molecular basis of CHD. Appropriate identification of cardiac disease, its epidemiology, and outcome is of utmost importance in guiding adequate care. Centralization of facilities is important to improve results and level of care

Absence of GJA1 gene mutations in four patients with anomalous left coronary artery from the pulmonary artery [ALCAPA]

Anomalous left coronary artery from the pulmonary artery [ALCAPA] is a rare form of congenital heart disease with no known genetic cause. It is usually diagnosed within the first year of life ; it results in myocardial cell death, congestive heart failure and lethality if left untreated. Connexin 43 alpha 1 is a member of the gap junction family of proteins demonstrated by animal studies to have a role in coronary artery patterning during development. No previous studies have investigated the role of this gene in patients with ALCAPA. We forth herein describe the clinical presentation of four patients who presented to the Children's Heart Center at AUBMC and had the final diagnosis of ALCAPA. Screening the GJA1 gene coding for connexin 43 alpha 1 was undertaken. No mutations were found in the patients or their parents, except for one polymorphism in one of the parents in the 3' untranslated region. All four patients underwent surgical repair with excellent outcome. This paper raises the awareness of this rare condition enabling physicians to reach the adequate diagnosis which will allow early surgical intervention and better prognosis. Our patient sample did not exhibit any evidence of association between connexin 43 alpha 1 and the clinical phenotype

Bilateral percutaneous stent implantation in the pulmonary vasculature: a modality now available in Lebanon

We report a child with Truncus Arteriosus who developed bilateral proximal branch pulmonary stenosis, following total surgical repair of her condition with construction of the right ventricular outflow tract utilizing a Contegra conduit [Medtronic. Inc., Minneapolis, Minn.]. The obstruction was relieved completely utilizing bilateral percutaneous stent implantation. To the best of our knowledge, this is the first reported case of bilateral stent implantation to relieve branch pulmonary artery stenosis in Lebanon. This technique could he applied to similar cases of obstruction in the pulmonary tree

Cardiac disease in children in Lebanon. the AUB-MC children's cardiac registry experience

To study the epidemiology of cardiac disease in children and their outcome in Lebanon, we established a Children Cardiac Registry Center [CCRC] at the American University of Beirut-Medical Center.design/methods: The CCRC included prospectively all pediatric patients with congenital heart disease [CHD] and/or acquired heart disease [AHD] who were evaluated at our center, between March 1,1997 and July 31,2000.Out of the 1000 patients with cardiac anomalies enrolled in the CCRC, 917 [91.7%] had CHD and the rest had AHD. Ventricular septal defect was the most common cardiac malformation with a relative frequency of 25.3%, followed by pulmonary stenosis [14.6%] aortic anomalies [8%], ASD [8%] and tetralogy of Fallot [7.8%]. Complex cardiac lesions like HL HS, TGA and AVC had lower frequencies at 0.4%, 3.7% and 3.5% respectively. The most common AHD was rheumatic heart disease [42.2%]. 34.9% of the registry patients with CHD and 10.8% with AHD underwent surgical intervention. There were 4.8% and 2.4% mortality rates in the CHD and AHD groups, respectively during the 40-month study period. The prevalence of many of the cardiac malformations in the CCRC was similar to that reported in the literature. However, some of the complex cardiac lesions were less common. The outcome of the two groups of patients is comparable to the outcome of children with cardiac malformation from developed countries. The establishment of a registry at the national level is important. Appropriate identification of the cardiac disease, its epidemiology, and outcome is of out most importance in guiding adequate care